Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

National Organization for Rare Disorders, Inc.

ImportantIt is possible that the main title of the report Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis is not the name you expected.

Disorder Subdivisions

None

General Discussion

SummaryStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent opposite ends of a spectrum of disease that results from an adverse reaction, most often to certain medications. SJS is the less severe end, but still represents a serious condition and potential medical emergency. TEN is a severe, life-threatening disorder. These disorders are differentiated by the degree of skin detachment. The consensus definition published in 1993 states that SJS affects less than 10% of the body surface area; TEN affects more than 30% of the body surface area. The term SJS/TEN-overlap syndrome is used to describe cases in which 10%-30% of the body surface area is detached. The reaction may start with a persistent fever and nonspecific, flu-like symptoms followed by appearance of erythematous macules (red spots) that may cover a large part of the body, and painful blistering of the skin and mucous membranes. The eyes are often involved. Numerous drugs have been reported to cause SJS and TEN and the following have shown an increased risk in larger studies: antibacterial sulfonamides, non-steroidal anti-inflammatory drugs of the oxicam type, certain anti-seizure drugs (antiepileptics), allopurinol and nevirapine. However, approximately one quarter (25%) of cases are not caused by drugs, but potentially by infections or have to be considered as idiopathic (of unknown cause).Individuals suspected of SJS or TEN should immediately stop taking the offending drug if it is known and all nonessential medications if it is not. Prompt recognition and early treatment are essential. It is also important to note that these disorders represent a spectrum of disease ranging from mild cases to those with severe, life-threatening complications. Consequently, every case is unique and the description of symptoms below will not apply to all individuals.

IntroductionSJS and TEN are classified as severe cutaneous adverse reactions (SCAR), a subcategory of adverse drug reactions (ADR). Unlike individuals with SJS and TEN, most individuals with a reactive skin disease have a mild and self-limiting condition. For years, confusing and contradictory terminology has been used to describe these disorders and controversy still exists as to the best way to classify them. In the past, erythema multiforme (EM) was considered part of this disease spectrum, but is now considered a distinct disorder. NORD has a separate report on erythema multiforme. These disorders are generally broken down into SJS, SJS/TEN overlap, and TEN. SJS was first described in the medical literature in 1922 by doctors A.M. Stevens and F.C. Johnson. The term toxic epidermal necrolysis was introduced in the medical literature in 1956 by Dr. A. Lyell and is also known as Lyell syndrome.

Supporting Organizations

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

Website: http://rarediseases.info.nih.gov/GARD/

NIH/National Institute of Allergy and Infectious Diseases

NIAID Office of Communications and Government Relations

5601 Fishers Lane, MSC 9806

Bethesda, MD 20892-9806

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

Email: ocpostoffice@niaid.nih.gov

Website: http://www.niaid.nih.gov/

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

Email: NIAMSinfo@mail.nih.gov

Website: http://www.niams.nih.gov/

Ocular Immunology and Uveitis Foundation

5 Cambridge Center

8th Floor

Cambridge, MA 2142

Tel: (617)621-6377

Fax: (781)431-2042

Tel: (866)353-6377

Email: fosters@comcast.net

Website: http://www.uveitis.org

Stevens Johnson Syndrome Foundation

PO Box 350333

Westminster, CO 80035-0333

Tel: (303)635-1241

Fax: (303)648-6686

Email: sjs@gmail.com

Website: http://www.sjsupport.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated: 7/14/2015Copyright 2015 National Organization for Rare Disorders, Inc.

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